Erişkinde Karaciğerin Diferansiye Olmamış Embriyonel Sarkomu (original) (raw)
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UNDIFERENTIATED EMBRYONIC SARCOMA OF THE LIVER: A CASE REPORT (ATENA EDITORA)
UNDIFERENTIATED EMBRYONIC SARCOMA OF THE LIVER: A CASE REPORT (ATENA EDITORA), 2022
Liver embryonic sarcoma (EHS) is a mesenchymal neoplasm of undetermined origin. This neoplasm is characterized by presenting solid and cystic components that may present points of necrosis or hemorrhage when in advanced stages. It is a malignant neoplasm, which affects in most cases children and adolescents between the first and second decade of life, with a peak incidence in children from five years of age. A good imaging exam associated with the clinical history (age at onset) and alpha-fetoprotein levels can help in the preoperative diagnosis. However, in clinical practice, especially when the disease affects another age group, we arrive at the diagnosis only through a post-surgical anatomopathological study. Among the differential diagnoses we can mention hemangioma, hepatoblastoma, hydatid cyst, hepatic cystadenoma and hepatic lymphangioma. Ultrasonography is the examination performed initially and helps in differentiating the components of the lesion; however, magnetic resonance imaging with the aid of liver-specific contrast (Primovist) is the gold standard for differentiating benign and malignant liver lesions, in addition to being essential for vascular assessment and involvement of adjacent structures, being essential to define the surgical strategy and resectability of the injury. This report describes the case of a 16-year-old female patient, who was admitted to the emergency room of our service due to vague and recurrent abdominal pain, associated with severe pain in the right shoulder and hyporexia. Patient was evaluated by our team, who chose to investigate the hospitalized patient. During hospitalization, magnetic resonance imaging of the abdomen was performed, which showed an oval hepatic subcapsular formation in segments VII and VIII of the right lobe and part of segment IVa. The lesion had thin walls, irregular internal membranes and possible signs of necrosis or recent hemorrhage. The lesion measured 14x12 cm and was in close contact with the diaphragm. In view of this finding, it was decided to perform a segmental hepatectomy of segments IV, V and VIII, associated with excision of the diaphragmatic portion adhered to the lesion. In the anatomopathological and immunohistochemical study of the surgical specimen, a malignant mesenchymal neoplasm of indeterminate histogenesis and characteristics compatible with hepatic embryonal sarcoma (Ki-67 of 40%) with free surgical margins were observed. The patient had a satisfactory postoperative evolution, being discharged on the seventh postoperative day and is currently being followed up by the clinical oncology team for complementary treatment and follow-up.
Embryonal Sarcoma of the Liver in the Adult: Challenges in the Diagnosis of a Rare Entity
GE - Portuguese Journal of Gastroenterology
Introduction: Embryonal sarcoma of the liver (ESL) is a rare neoplasm of the liver occurring mainly in paediatric ages. Making the correct diagnosis can be challenging as the laboratory and radiological findings that are often nonspecific, and the tumour immunophenotype is poorly defined and even somewhat variable. Case Presentation: A large epigastric mass was detected in a computerized tomography scan of a 43-year-old woman presenting with abdominal pain and bloating. The mass was biopsied and submitted to histopathological study. Microscopically the tumour had sarcomatoid features and showed multinucleated cells with periodic acid-Schiff (PAS)-positive globules. Immunostaining revealed positivity for vimentin, CD10, glypican-3, and α1-antitrypsin and negativity for keratins, muscle, adipocytic, and melanocytic differentiation markers. The patient was then submitted to a left hepatectomy with similar histological findings. Discussion: ESL in adults is a rarity and its diagnosis re...
Undifferentiated Embryonal Sarcoma of the Liver: A Concise Review
Archives of Pathology & Laboratory Medicine, 2015
Undifferentiated embryonal sarcoma of the liver is an aggressive mesenchymal tumor that occurs predominantly in children. Although this entity has been described for decades, its pathogenesis is still obscure. Its association with mesenchymal hamartoma has been well described on the basis of identical chromosomal abnormalities. The clinical and radiological diagnoses are often difficult, and the diagnosis of undifferentiated embryonal sarcoma of the liver is based on its histology and immunophenotype. It is essential to recognize the characteristic histologic findings and the pattern of the immunohistochemistry staining to rule out other hepatic lesions. Multimodal therapy with surgery, chemotherapy, and radiation therapy has drastically improved the prognosis of patients with undifferentiated embryonal sarcoma of the liver. This successful management requires timely diagnosis for superior outcome.
Diagnostic and Interventional Imaging, 2012
Undifferentiated (embryonal) sarcoma; Liver; Adult; Anatomical/pathological correlation Mrs. V, a 61-year-old woman with no particular history, was seen for impairment of general health and abdominal pain. The physical examination found a tender mass in the right hypochondrium. The laboratory tests revealed the presence of inflammation (CRP: 350 mg/L, 15,000 neutrophils), cytolysis (ALT: 130 IU/L, AST: 150 IU/L: 4 N), anicteric cholestasis (alkaline phosphatase: 450 IU/L); tumor markers AFP (8 ng/mL) and CA 125 (27 U/mL) were normal.
Annals of Diagnostic Pathology, 2011
Undifferentiated (embryonal) liver sarcoma (UELS) is a rare tumor that typically occurs in children. The association of UELS with neoplasm other than mesenchymal liver hamartoma is exceedingly rare. The aim of the study was to report 3 cases of UELS, 2 of them being interesting because of their association with another neoplasm, vaginal embryonal rhabdomyosarcoma in a teenage girl and Bacute lymphoblastic leukemia in a middle-aged woman. Besides, one of our cases of UELS, in a 58year-old woman, is an extremely rare presentation of such a tumor in a middle-aged adult. The patient's clinical features, therapy, and pathologic results were reviewed; immunohistochemical analysis and, in 2 cases, electron microscopy were performed. In this study, all 3 patients were females aged 13, 13, and 58 years. Histopathologic evaluation of resected liver tumors confirmed the diagnosis of UELS in all of them. In 2 of the cases, metachronous occurrence of UELS with vaginal embryonal rhabdomyosarcoma in a teenage girl and B-acute lymphoblastic leukemia in a middleaged woman is described. Careful clinical analysis, histologic studies, and immunohistochemistry are mandatory to distinguish UELS from other hepatic malignancies with similar or overlapping features and to exclude the possibility of other tumors that may be considered in the differential diagnosis. The association of UELS with another neoplasm is exceedingly rare.
Undifferentiated embryonal sarcoma of liver: Paradoxical imaging appearance
Radiology Case Reports, 2020
Undifferentiated embryonal sarcoma of the liver is a rare entity. It is a malignant primitive mesenchymal tumor seen in the pediatric age group often between 6 to 10 years of age. It involves the right lobe of the liver commonly and is usually asymptomatic. Acute presentation in these cases is secondary to its rupture/ wall dehiscence. Alfa fetoprotein, a tumor marker elevated in most of the hepatic malignant tumors is however normal in undifferentiated embryonal sarcoma. Imaging wise it is a large encapsulated multiseptated lesion. It shows a “paradoxical appearance” with a predominantly solid appearance on ultrasonography and cystic appearance on CT/MRI. This is a peculiar feature that can help in the early diagnosis of this entity. Besides, normal serum alfa fetoprotein levels favor its diagnosis. Hereby we present a case of a 5-year-old female child, presented with complaints of acute onset abdominal pain and distension which on imaging investigation showed a liver mass with typ...
Unexpected Liver Embryonal Sarcoma in the Adult: Diagnosis and Treatment
Case reports in surgery, 2018
Undifferentiated embryonal sarcoma of the liver is a malignancy with poor prognosis observed more frequently in children between 6 and 10 years old and very rarely found in adults. We present a case of embryonal sarcoma of the liver in a 60-year-old woman without significant medical history who presented to our attention with constitutional symptoms. Preoperative assessments did not show alterations in blood chemistry or tumor markers. Imaging studies showed a huge mass lying in the right abdominal quadrants, strictly adherent to the liver. The tumor was partially cystic with a thickened wall, sporadic contrast enhancement, and solid component. The patient underwent excision of the mass with associated liver bisegmentectomy S5-S6. Postoperative course was uneventful. The definitive histological diagnosis revealed the presence of embryonal sarcoma of the liver. We describe the clinical, histopathological, and therapeutic options adopted in the multimodal treatment of this disease.
Embryonal sarcoma of the liver
Pediatric Surgery International, 2013
Purpose Embryonal sarcoma accounts for 6 % of liver tumors. This study reviews its features and the results of treatment in a referral center. Methods We retrospectively reviewed liver tumors treated between 1995 and 2011. PRETEXT staging and biopsy were used to tailor chemotherapy according to SIOP protocols. Radical surgery was performed when possible. Complications and cumulative survival were the outcome endpoints. Results Six out of 156 primary liver tumors (four males and two females) were sarcomas. The mean age at diagnosis was 81 ± 8.5 months. The most frequent finding was abdominal mass. Alfa-fetoprotein was normal. Imaging depicted heterogeneous tumors with septa, necrosis, and hemorrhagic areas. The diagnosis was ascertained by biopsy. Three tumors were located in the right lobe (PRETEXT II): two were bilobar (PRETEXT III) and one was in the left lobe (PRETEXT I). Two children had metastases at diagnosis and high-risk chemotherapy (vincristine, carboplatin, epirubicin) was administered with poor response. They died without operation 4 and 10 months later. Four patients with local disease underwent typical liver resections after chemotherapy (iphosphamide, vincristine, actinomycin D, and doxorrubicin). Overall actuarial survival at 70 months was 66.6 %. Conclusions Extended and metastatic embryonal sarcoma do poorly whereas localized tumors amenable to complete surgical removal after chemotherapy can cure.